Background
Pemphigus encompasses a group of life‐threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, pemphigus was almost always fatal. Due to its rarity, only few randomized controlled therapeutic trials are available. Recently, rituximab has been approved as first‐line treatment for moderate and severe pemphigus vulgaris in Europe and the United States.
Objectives
The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology (EADV) has initiated a throughout update of the guideline for the management of patients with pemphigus.
Results
The guidelines for the management of pemphigus were updated, and the degree of consent among all task force members was included. The final version of the guideline was consented by the European Dermatology Forum (EDF) and several patient organizations.
Background Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo-like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life.
Objectives and methodologyThe Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology sought to update the guidelines for the management of BP based on new clinical information, and new evidence on diagnostic tools and interventions. The recommendations are either evidence-based or rely on expert opinion. The degree of consent among all task force members was included.Results Treatment depends on the severity of BP and patients' comorbidities. High-potency topical corticosteroids are recommended as the mainstay of treatment whenever possible. Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of contraindications or resistance to corticosteroids, immunosuppressive therapies, such as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be recommended. The use of doxycycline and dapsone is controversial. They may be recommended, in particular, in patients with contraindications to oral corticosteroids. B-cell-depleting therapy and intravenous immunoglobulins may be considered in treatment-resistant cases. Omalizumab and dupilumab have recently shown promising results. The final version of the guideline was consented to by several patient organizations.
ConclusionsThe guidelines for the management of BP were updated. They summarize evidence-and expert-based recommendations useful in clinical practice.
NB-UVB performed better in comparison to TMP PUVA in terms of mean total repigmentation when traditionally considered therapy-resistant sites were excluded.
Well-designed studies on systemic therapeutic modalities for severe psoriasis in children are rare. Children with severe disease are treated with the support of data extrapolated from that in adult, although management in them differs from adults in several important aspects. Like other systemic modalities, data regarding the use of methotrexate in the treatment of childhood psoriasis is meager. This study aims to analyze the efficacy and safety of methotrexate in severe or disabling childhood psoriasis. The records of all the patients <18 years of age treated with systemic methotrexate at the psoriasis clinic of our institute from January 1993 to December 2006 were retrieved. Information regarding demographic profile, disease characteristics, response to treatment, side effects, etc. was noted from predesigned clinic proforma. Indications of methotrexate use were baseline psoriasis area and severity index (PASI) >10, disease refractory to conventional therapies and disabling psoriasis even though the psoriasis area and severity index was <10. Clinical status of patients was assessed at weekly intervals for the first 2 weeks, fortnightly during next month and then monthly. Response to therapy was graded as good (50-75% decrease in PASI) and excellent (>75% decrease in PASI). Laboratory investigations to detect methotrexate induced toxicity were performed at regular intervals. Of the 29 patients treated with methotrexate, 24 were eligible for the final data analysis. Indication for the institution of methotrexate therapy was severe disease, viz., extensive recalcitrant plaque type psoriasis in 17 patients, erythroderma and generalized pustular psoriasis of von-Zumbusch type in three patients each and severe disabling palmo-plantar involvement along with chronic plaque lesions in one patient. Response to therapy was excellent (>75% decrease in PASI) in all but two patients. The mean time to control the disease, i.e., 50% reduction in PASI was 5.1 weeks. Mean total cumulative dose of methotrexate in the first episode was 215 mg. The duration of remission could be calculated in nine patients only, varying from 1.5 months to 3 years. Side effects were mild, observed in nine children, which included nausea, vomiting, and loss of appetite. Methotrexate is an effective, cheap, easily available, and reasonably safe drug to be used in severe childhood psoriasis under an expert supervision and laboratory monitoring.
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